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Mds 1
- 3. Definition Clonal hematopoietic stem cell disorders characterized by cytopenias, dysplasia in one or more of the major myeloid cell lines, ineffective hematopoiesis with cellular marrow and risk for leukemic transformation.
- 4. 1. Bone marrow cannot produce blood cells effectively 2. If produced the blood cells will be defective in nature 3. These abnormal cells usually destroyed before they leave from bone marrow or shortly after entering into the blood stream
- 5. 1. Varying degree of tri lineage cytopenias 2. Dysplasia 3. Normocellular or Hypercellular Bone marrow 4. May progress to acute leukemia's
- 6. Incidence 1. Disease of elderly 2. Median age is 65 years 3. M>F 4. Incidence 1/1000/years > 60 years
- 7. PREDISPOSING FACTORS HEREDITARY 1. Down’s syndrome 2. NF 3. Congenital Neutropenia 4. Fanconi’s anemia 5. Shwachman Diamond syndrome 6. Diamond Blackfan syndrome ACQUIRED 1. Radiation 2. Mutation 3. Alkylating agents 4. Tabacoo 5. Benzene 6. Aplastic anemia 7. PNH
- 9. FAB CLASSIFIACTION SUB TYPE PS BM RA Blasts < 1% Blasts < 5% RS < 15% RA – RS Blasts < 1% Blasts < 5% RS > 15% RAEB Blasts < 5% Blasts 5-20% RAEBT Blasts > 5% Blasts 20-30% Auer rods CMML Blasts < 5% Monocyte > 1% Any of the above AML Variable Blasts > 30%
- 10. WHO CLASSIFICATION SUBTYPE PS BM RCUD Unicytopenia / No blast Unilineage dysplasia > 10% of cells Blasts < 5% RS < 15% RARS Anemia Erythroid hyperplasia with Dyserythropoiesis RS > 15% Blasts < 5% RCMD Bi / Pancytopenia No blast / No Auer rods Dysplasia >10 % in > 2 lineage Blast <5% RS > 15% RAEB-1 <5% blasts Bi / Pancytopenia No Auer Rods 5-9% blasts No Auer rods Dysplasia > 1 hematopoietic cell line RAEB-2 5-9% blasts Cytopenias Auer rods 10-19% blasts Auer rod + Myelodysplasia > 1 line
- 11. MDS – U No blasts Cytopenias only < 5% blasts Cytogenetic abnormalities Granulocytic or Megakaryocytic dysplasia MDS – 5q deletion < 1% blasts Anemia Increased platelets < 5% blasts Isolated 5q deletion No Auer rods CMDS < 2 % blasts Dysplastic changes in > 10 % of neutrophils Dysplasia > 10% erythroid precursors Dysplasia > 10% granulocytic precursors
- 12. Etio pathogenesis 1. Denovo 2. Therapy related
- 13. Characteristic features 1. Dyserythropoiesis 2. Dysmyelopoiesis 3. Dysmegakaryopoiesis 4. Ring sideroblast 5. Type I & II blast
- 14. PERIPHERAL SMEAR Erythroid • Ovalocytosis • Macrocytosis • Eliptocytosis • Tear drop cells • Nucleated red cells • Basophilic stippling • Howell –Jolly bodies Myeloid • Pseudo – Pelger Huet anomaly • Auer rod • Hypogranulation • Hypersegmentation of nuclei Megakaryocyte • Giant platelets • Hypogranular platelets • Agranullar platelets
- 15. Bone marrow Erythroid • Megaloblastic erythropoiesis • Nuclear budding • Ring sideroblast • Internuclear bridging • Nuclear fragments • Multinucleation Myeloid • Defective granulation • Hypolobation • Auer rods • Maturation arrest • ALIP Megakaryocyte • Micromegakaryocyte • Hypogranulation • Hypolobation • Multiple small nuclei
- 19. Clinical presentation 1. Anemia 2. Lethargy 3. Weakness 4. Fainting 5. Palpitation 6. Dizziness 7. Headache 8. Menstrual abnormalities 9. Poor appetite 10.Irritability
- 23. Treatment 1. Supportive therapy 2. Chemotherapy 3. Bone marrow transplantation